Cardiolipin remodeling by TAZ/tafazzin is selectively required for the initiation of mitophagy

摘要: Tafazzin (TAZ) is a phospholipid transacylase that catalyzes the remodeling of cardiolipin, mitochondrial required for oxidative phosphorylation. Mutations TAZ cause Barth syndrome, which characterized by dysfunction and dilated cardiomyopathy, leading to premature death. However, molecular mechanisms underlying in syndrome remain poorly understood. Here we investigated role regulating function mitophagy. Using primary mouse embryonic fibroblasts (MEFs) with doxycycline-inducible knockdown Taz, showed deficiency MEFs caused defective mitophagosome biogenesis, but not other autophagic processes. Consistent key mitophagy mitochondria quality control, also led impaired phosphorylation severe stress. Together, these findings provide insights on suggesting pharmacological restoration may novel treatment this lethal condition.